Multimodal imaging of foveoschisis and macular pseudohole associated with gyrate atrophy: a family report

Abstract Background To report the results of multimodal imaging of a biochemically confirmed case of a family with gyrate atrophy (GA) associated with foveoschisis and macular pseudohole. Case presentation Two sisters presented to us with progressive bilateral decreased vision. The 26-year old sister had a best corrected visual acuity (BCVA) of 20/32 in the right eye (RE) and 20/100 in the left eye (LE). Dilated fundus examination revealed multiple bilateral chorioretinal atrophy areas in the midperipheral and peripheral retina. Fluorescein angiography did not show any leak in the macular area. Swept-source optical coherence tomography (SS-OCT) showed increased central macular thickness in both eyes with foveoschisis. Optical coherence tomography angiography (OCTA) showed petaloid non-reflective areas and some perifoveal microvascular alterations similar to telangiectasias in the deep capillary complex. The 30-year-old sister had a BCVA of 20/20 in the RE and 20/32 in the LE. SS-OCT was normal in the RE and demonstrated a macular pseudohole with a fine epiretinal membrane in the LE. The persistent retinal tissue at the base of the pseudohole was disorganised. Blood tests showed hyperornithinemia in the 2 cases. Based on these observations, the patients were diagnosed with gyrate atrophy of the choroid and retina and were treated with a pyridoxine supplement and an arginine-restricted diet. Conclusions Foveoschisis and macular pseudohole may be associated in GA, increasing the risk of rapid vision loss. OCTA is an interesting imaging tool that can help to better understand the pathophysiological mechanism of these macular involvements in GA

The International Council of Ophthalmology Ophthalmic clinical evaluation exercise

PURPOSE: Fifteen years after the publication of the Ophthalmic Clinical Evaluation Exercise (OCEX), it was deemed necessary to review and revise it, and to validate it for an international audience of ophthalmologists. This study to revise the OCEX and validate it for international use. METHODS: The OCEX rubric was changed to a modified Dreyfus scale; a behavioral descriptor was created for each category. An international panel of ophthalmic educators reviewed the international applicability and appropriateness of the tool. RESULTS: A tool for assessing and giving feedback on four aspects of clinical competence during the ophthalmic consultation (interview skills, examination, interpersonal and communication skills, and case presentation) was revised. The original scoring tool was improved to a new behavioral one, and relevant comments and suggestions from international reviewers were incorporated. The new tool has face and content validity for an international audience. CONCLUSION: The OCEX is the only tool for workplace assessment and feedback specifically for ophthalmology residents and the ophthalmic consultation. This improved and simplified version will facilitate its use and implementation to diverse programs around the world